Mattia Zanin*, Enrico Bonaveglio, Martina Scanu, Annalisa Longobardo, Stefania Iannandrea, Elisa Catalano, Fortunato Ciliberto F, Sergio Grassitelli, Francesca Fusco, Mirela Bojan and Simona Quaglia
Pulmonary Arterial Hypertension (PAH) is a well-known but very rare and severe complication of Incontinentia Pigmenti (IP). Only few cases are reported in literature, most of them fatal. Recently, some reports described better outcome with a more aggressive treatment combining multiple pulmonary vasodilators and immunomodulatory drugs.
We present the case of a three months old female diagnosed with IP and severe PAH refractory to inhaled nitric oxyde, admitted in cardio-pediatric ICU for mixed cardiogenic and hemorrhagic shock and treated with a combination of high dose iNO, sildenafil, iloprost, TNF-alpha inhibitor and steroids. Following this treatment, PAP dropped from iso-systemic to half systemic, and PVRI halved as well. After an episode of septic shock and a hospital acquired pneumopathy from Pneumocystis jirovecii, the baby was discharged home in good conditions at the age of six months.
Published Date: 2022-09-27; Received Date: 2022-08-30
